PMID: 6447771Jul 1, 1980

Metabolic impairment and membrane abnormality in red cells from Huntington's disease

Journal of the Neurological Sciences
A ZanellaG Scarlato

Abstract

The following aspects have been investigated in 10 patients affected by Huntington's disease )HD): --extensive haematological investigations; --red cell enzyme activities and level of the most important glycolytic intermediate compounds; --protein, lipid and carbohydrate composition of the erythrocyte membrane and membrane polarity; --effects of in vitro aging on red cell membranes. Lack of 4.5 protein band in SDS-PAGE and 14-fold decrease in membrane-bound catalase were found in the in vitro aged red cells from the 10 HD patients examined. Na+ + K+ATPase was slightly higher than normal in all the patients. Red cells from 5 out of 8 patients showed a decrease in reduced glutathione and phosphoenolpyruvate levels and/or an increase in hexokinase, glucose-6-phosphate dehydrogenase, pyruvate kinase and glutathione reductase activities. The haematological investigations, the protein lipid and carbohydrate composition of the fresh red cells, the membrane polypeptide aggregates and the membrane polarity evaluated by microspectrofluorometric analysis were normal.

References

Aug 19, 1976·The New England Journal of Medicine·E Beutler
Sep 23, 1977·Biochemical and Biophysical Research Communications·W R Markesbery, D A Butterfield
Oct 15, 1977·Life Sciences·T D ReisineH I Yamamura
Sep 1, 1977·Life Sciences·K G LloydE D Bird
May 1, 1979·British Journal of Haematology·A ZanellaC Balduini
Dec 1, 1978·British Journal of Haematology·A ZanellaG Sirchia
Jan 1, 1978·American Journal of Hematology·A ZanellaG Sirchia
Mar 1, 1976·British Journal of Haematology·A ZanellaG Sirchia
Feb 1, 1974·Analytical Biochemistry·W Niedermeier, M Tomana
Apr 19, 1973·The New England Journal of Medicine·J H Menkes, N Stein
Oct 31, 1973·Nature: New Biology·P G RighettiG Sirchia
Jul 1, 1974·The Journal of Cell Biology·T L Steck
Mar 1, 1970·British Journal of Haematology·G P CanellosJ V Dacie
Jan 1, 1963·Archives of Biochemistry and Biophysics·J T DODGED J HANAHAN

Citations

Mar 1, 1982·Journal of the Neurological Sciences·R M Mangano, R Schwarcz
Jul 20, 1982·Neuroscience Letters·G SarpelA Omachi
Dec 22, 2004·Proceedings of the National Academy of Sciences of the United States of America·Marcus J CalkinsJeffrey A Johnson
Sep 7, 2013·Toxicological Sciences : an Official Journal of the Society of Toxicology·Márcio RibeiroA Cristina Rego
Apr 2, 2010·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Xueyi LiMarian Difiglia
Jun 1, 1982·Journal of Neurochemistry·C Sumbilla, J R Lakowicz
May 1, 1982·Acta Neurologica Scandinavica·G C Beverstock, P L Pearson
May 1, 1984·American Journal of Medical Genetics·M K McCormackF Lepore
Jan 1, 1982·American Journal of Medical Genetics·M K McCormackA Lazzarini
Apr 5, 2012·BioFactors·M Alba SorollaElisa Cabiscol
Jan 1, 1985·Clinical Genetics·L A Farrer
May 28, 2019·Journal of Cellular Biochemistry·Bor Luen Tang

Related Concepts

Acetylthiocholinesterase
DNA-dependent ATPase
Catalase T
Erythrocyte Survival
Red Cell Ghost
Erythrocytes
Glyceraldehyde-3-Phosphate Dehydrogenases
Glycolysis
Akinetic-Rigid Variant of Huntington Disease
Lactate Dehydrogenase

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